Outcomes of Children With Cardiomyopathy Listed for Transplant

A Multi-institutional Study

Anne I. Dipchand, David C. Naftel, Brian Feingold, Robert L Spicer, Delphine Yung, Beth Kaufman, James K. Kirklin, Tina Allain-Rooney, Daphne Hsu

Research output: Contribution to journalArticle

45 Citations (Scopus)

Abstract

Background: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined. Methods: A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%). Results: CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001). Conclusion: Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.

Original languageEnglish (US)
Pages (from-to)1312-1321
Number of pages10
JournalJournal of Heart and Lung Transplantation
Volume28
Issue number12
DOIs
StatePublished - Dec 1 2009

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Cardiomyopathies
Transplants
Survival
Artificial Respiration
Mortality
Extracorporeal Membrane Oxygenation
Hypertrophic Cardiomyopathy
Heart Transplantation
Registries

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

Cite this

Outcomes of Children With Cardiomyopathy Listed for Transplant : A Multi-institutional Study. / Dipchand, Anne I.; Naftel, David C.; Feingold, Brian; Spicer, Robert L; Yung, Delphine; Kaufman, Beth; Kirklin, James K.; Allain-Rooney, Tina; Hsu, Daphne.

In: Journal of Heart and Lung Transplantation, Vol. 28, No. 12, 01.12.2009, p. 1312-1321.

Research output: Contribution to journalArticle

Dipchand, AI, Naftel, DC, Feingold, B, Spicer, RL, Yung, D, Kaufman, B, Kirklin, JK, Allain-Rooney, T & Hsu, D 2009, 'Outcomes of Children With Cardiomyopathy Listed for Transplant: A Multi-institutional Study', Journal of Heart and Lung Transplantation, vol. 28, no. 12, pp. 1312-1321. https://doi.org/10.1016/j.healun.2009.05.019
Dipchand, Anne I. ; Naftel, David C. ; Feingold, Brian ; Spicer, Robert L ; Yung, Delphine ; Kaufman, Beth ; Kirklin, James K. ; Allain-Rooney, Tina ; Hsu, Daphne. / Outcomes of Children With Cardiomyopathy Listed for Transplant : A Multi-institutional Study. In: Journal of Heart and Lung Transplantation. 2009 ; Vol. 28, No. 12. pp. 1312-1321.
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abstract = "Background: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined. Methods: A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42{\%}) and 1,827 with non-CM (58{\%}) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83{\%}), 145 RCM (11{\%}), and 77 HCM (6{\%}). Results: CM patients had a waitlist mortality of 17{\%} vs 32{\%} for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66{\%} for CM vs 53{\%} for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68{\%} vs 61{\%}, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001). Conclusion: Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.",
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T1 - Outcomes of Children With Cardiomyopathy Listed for Transplant

T2 - A Multi-institutional Study

AU - Dipchand, Anne I.

AU - Naftel, David C.

AU - Feingold, Brian

AU - Spicer, Robert L

AU - Yung, Delphine

AU - Kaufman, Beth

AU - Kirklin, James K.

AU - Allain-Rooney, Tina

AU - Hsu, Daphne

PY - 2009/12/1

Y1 - 2009/12/1

N2 - Background: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined. Methods: A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%). Results: CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001). Conclusion: Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.

AB - Background: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined. Methods: A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%). Results: CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001). Conclusion: Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.

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